中华血液学杂志
中華血液學雜誌
중화혈액학잡지
Chinese Journal of Hematology
2009年
3期
179-182
,共4页
赵馨%周康%王慧君%张莉%刘庆国%井丽萍%李洪强%杨栋林%储榆林%张风奎
趙馨%週康%王慧君%張莉%劉慶國%井麗萍%李洪彊%楊棟林%儲榆林%張風奎
조형%주강%왕혜군%장리%류경국%정려평%리홍강%양동림%저유림%장풍규
白血病,大颗粒淋巴细胞%贫血,再生障碍,纯红细胞%免疫抑制治疗
白血病,大顆粒淋巴細胞%貧血,再生障礙,純紅細胞%免疫抑製治療
백혈병,대과립림파세포%빈혈,재생장애,순홍세포%면역억제치료
Leukemia,T cell large granular lymphocytic%Pure red cell aplasia%Immunosup pressive therapy
目的 提高对T大颗粒淋巴细胞白血病(T-LGLL)的认识.方法 回顾性分析我院1999年6月至2007年7月间确诊的27例T-LGLL患者临床及实验室检查特征.结果 T-LGLL起病潜隐、进展缓慢,中位确诊年龄48岁.贫血相关症状最为突出,51.9%患者脾脏轻中度肿大,14.8%患者肝脏轻度肿大.未有合并类风湿性关节炎者.外周血中性粒细胞＜1.5×109/L者19例,占70.4%,＜0.5×109/L者5例,占18.5%.贫血占88.9%,中位血红蛋白58 g/L,合并纯红细胞再生障碍者18例,占66.67%.外周血大颗粒淋巴细胞(LGL)绝对值中位数1.45×109/L,21例(77.8%)患者LGL数≤2.0×109/L,22例(81.5%)患者LGL免疫表型为CD3+CD8+CD57+CD56-.免疫抑制治疗总有效率91.3%,完全血液学缓解率为65.2%.结论 T-LGLL起病及进展缓慢,主要表现为血细胞减少和脾脏肿大,常合并纯红细胞再生障碍.多数患者外周血LGL计数≤2.0×109/L,免疫表型以CD3+CD8+CD57+为主,对免疫抑制治疗反应良好.
目的 提高對T大顆粒淋巴細胞白血病(T-LGLL)的認識.方法 迴顧性分析我院1999年6月至2007年7月間確診的27例T-LGLL患者臨床及實驗室檢查特徵.結果 T-LGLL起病潛隱、進展緩慢,中位確診年齡48歲.貧血相關癥狀最為突齣,51.9%患者脾髒輕中度腫大,14.8%患者肝髒輕度腫大.未有閤併類風濕性關節炎者.外週血中性粒細胞＜1.5×109/L者19例,佔70.4%,＜0.5×109/L者5例,佔18.5%.貧血佔88.9%,中位血紅蛋白58 g/L,閤併純紅細胞再生障礙者18例,佔66.67%.外週血大顆粒淋巴細胞(LGL)絕對值中位數1.45×109/L,21例(77.8%)患者LGL數≤2.0×109/L,22例(81.5%)患者LGL免疫錶型為CD3+CD8+CD57+CD56-.免疫抑製治療總有效率91.3%,完全血液學緩解率為65.2%.結論 T-LGLL起病及進展緩慢,主要錶現為血細胞減少和脾髒腫大,常閤併純紅細胞再生障礙.多數患者外週血LGL計數≤2.0×109/L,免疫錶型以CD3+CD8+CD57+為主,對免疫抑製治療反應良好.
목적 제고대T대과립림파세포백혈병(T-LGLL)적인식.방법 회고성분석아원1999년6월지2007년7월간학진적27례T-LGLL환자림상급실험실검사특정.결과 T-LGLL기병잠은、진전완만,중위학진년령48세.빈혈상관증상최위돌출,51.9%환자비장경중도종대,14.8%환자간장경도종대.미유합병류풍습성관절염자.외주혈중성립세포＜1.5×109/L자19례,점70.4%,＜0.5×109/L자5례,점18.5%.빈혈점88.9%,중위혈홍단백58 g/L,합병순홍세포재생장애자18례,점66.67%.외주혈대과립림파세포(LGL)절대치중위수1.45×109/L,21례(77.8%)환자LGL수≤2.0×109/L,22례(81.5%)환자LGL면역표형위CD3+CD8+CD57+CD56-.면역억제치료총유효솔91.3%,완전혈액학완해솔위65.2%.결론 T-LGLL기병급진전완만,주요표현위혈세포감소화비장종대,상합병순홍세포재생장애.다수환자외주혈LGL계수≤2.0×109/L,면역표형이CD3+CD8+CD57+위주,대면역억제치료반응량호.
Objective To analyze the characteristics of T-cell large granular lymphocyte leukemia (T-LGLL).Methods Retrospectively analyze the clinical and laboratory data of 27 patients with T-LGLL diagnosed between 1999 and 2007 in our hospital.Results The median age at diagnosis was 48 years.All patients were symptomatic,mainly complaining of fatigue.Of the 27 patients,14(51.9%)had splenomegaly,and 4(14.8%)hepatomegaly.Rheumatoid arthritis Was not present in any patients.The most frequent hematological abnormality was anemia(24 patients,88.9%)with a median Hb level of 57.5g/L.Pure red cell aplasia Was found in 18 patients(66.67%).The median WBC count was 4.24×109/L and 19 cases were neutropenia(ANC＜1.5×109/L).The median LGL count in peripheral blood was 1.45×109/L and most of them(77.8%)were less than 2.0×109/L.Twenty-two patients(81.5%)showed the CD3+CD8+CD57+CD56-LGL phenotype.With immunosuppressive thempy.91.3%of patients responded and complete hematological remission rate was 65.2%.Conclusion T-LGLL mainly presented with anemia and complete hematological remission rate was 65.2%.Pure red cell aplasia WaS commonly associated with the disease.The patients had a good response to immunesuppressive therapy.
